Coronary Artery Changes in Patients with Multisystem Inflammatory Syndrome in Children: Los Angeles Experience.

We compared cardiac findings in patients with multisystem inflammatory syndrome in children and Kawasaki disease in the first 6 months of the 2020 coronavirus disease pandemic to patients with Kawasaki disease during 2016-2019. We saw a high rate of coronary aneurysms in 2020, with a similar rate of coronary involvement but greater volume and incidence of cardiac dysfunction compared with previous years.

Third Trimester Fetal Heart Rates in Antibody-Mediated Complete Heart Block Predict Need for Neonatal Pacemaker Placement.

Congenital complete heart block (CCHB) affects 1 in 20,000 newborns. This study evaluates fetal and neonatal risk factors predictive of neonatal pacemaker placement in antibody-mediated complete heart block. The Children's Hospital Los Angeles institutional fetal, pacemaker, and medical record databases were queried for confirmed SSA/SSB cases of CCHB between January 2004 and July 2019. Cases excluded were those with a diagnosis beyond the neonatal period, diagnosis of a channelopathy, or if maternal antibody status was unknown. We recorded the gestational age (GA), birth weight (BW), fetal heart rates (FHRs) of the last echocardiogram before delivery, specific neonatal ECG and echocardiogram findings, age at pacemaker placement, and mortality. Of 43 neonates identified with CCHB, 27 had confirmed maternal antibody exposure. Variables associated with neonatal pacemaker implantation were FHRs < 50 bpm (p = 0.005), neonatal heart rates < 52 bpm (p = 0.015), and neonatal left ventricular fractional shortening (FS) percentages < 34% (p = 0.03). On multivariate analysis, FHR remained significant (p = 0.03) and demonstrated an increased risk of neonatal pacemaker placement by an odds ratio of 12.5 (95% CI 1.3-116, p = 0.05). The median GA at which the FHR was obtained was 34 weeks (IQR 26-35 weeks). Neonatal pacemaker placement was highly associated with a FHR < 50 bpm, neonatal HR < 52 bpm, and neonatal FS < 34%. FHRs at 34 weeks GA (IQR 26-35 weeks) correlated well with postnatal heart rates and were predictive of neonatal pacemaker placement.

Shear Stress Causing Hemolytic Anemia and Congestive Heart Failure as an Initial Presentation of Cor Triatriatum in an Infant.

Among congenital cardiac defects, cor triatriatum sinistrum is a rare formation of 3 distinct atrial chambers in the heart. Depending on the size of the defect in the membranous septum, the symptoms can range from asymptomatic to severe pulmonary hypertension. Individuals can go years, sometimes to the second and third decades of life, before presenting with symptoms. Although it is well known to be associated with pulmonary veno-occlusive disease, the association with hemolytic anemia is much less well known. Identifying the subtle signs and symptoms of cor triatriatum in the emergency department can save a life. Here, we present the case of a 6-month-old infant with hemolytic anemia in the setting of right-sided congestive heart failure leading to the diagnosis of cor triatriatum. Anemia developed secondary to shearing stress caused by the heart defect itself. Complete resolution of symptoms occurred status post septectomy and repair of the defect.

Rapidly progressive heart failure requiring transplantation in muscular dystrophy: a need for frequent screening.

Fukuyama congenital muscular dystrophy weakens both skeletal and cardiac muscles, but the rate of cardiomyopathic progression can accelerate faster than that of skeletal muscles. A 14-year-old boy with Fukuyama congenital muscular dystrophy presented with mild skeletal myopathy but severe cardiomyopathy requiring heart transplantation within 1 year of declining heart function. These patients need frequent screening regardless of musculoskeletal symptoms.

Implantable cardioverter-defibrillator implantation for primary and secondary prevention: indications and outcomes.

Implantable cardioverter-defibrillators effectively reduce the rate of sudden cardiac death in children. Significant efforts have been made to better characterise the indications for their placement, and over the past two decades there has been a shift in their use from secondary to primary prevention. Primary prevention includes placement in patients thought to be at high risk of sudden cardiac death before the patient experiences any event. Secondary prevention includes placement after a high-risk event including sustained ventricular tachycardia or resuscitated cardiac arrest. Although liberal device implantation may be appealing even in patients having marginal indications, studies have shown high rates of adverse effects including inappropriate device discharges and the need for re-intervention because of hardware malfunction. The indications for placement of an implantable cardioverter-defibrillator, whether for primary or secondary prevention of sudden cardiac death, vary based on cardiac pathology. This review will assist the provider in understanding the risks and benefits of device implantation in order to enhance the shared decision-making capacity of patients, families, and providers.